Systemic Sclerosis

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The three-year incidence of pulmonary arterial hypertension associated with systemic sclerosis in a multicenter nationwide longitudinal study in France

  1. Eric Hachulla1,†,*,
  2. Pascal de Groote2,
  3. Virginie Gressin3,‡,
  4. Jean Sibilia4,§,
  5. Elisabeth Diot5,
  6. Patrick Carpentier6,¶,
  7. Luc Mouthon7,‖,
  8. Pierre-Yves Hatron1,††,
  9. Patrick Jego8,
  10. Yannick Allanore7,‡‡,
  11. Kiet Phong Tiev9,
  12. Christian Agard10,
  13. Anne Cosnes11,
  14. Daniela Cirstea12,
  15. Joël Constans13,
  16. Dominique Farge14,
  17. Jean-François Viallard15,
  18. Jean-Robert Harle16,
  19. Frédéric Patat17,
  20. Bernard Imbert6,
  21. André Kahan7,§§,
  22. Jean Cabane9,
  23. Pierre Clerson18,
  24. Loïc Guillevin7,¶¶,
  25. Marc Humbert19

Article first published online: 28 MAY 2009

DOI: 10.1002/art.24525

Issue

Arthritis & Rheumatism

Arthritis & Rheumatism

Volume 60, Issue 6, pages 1831–1839, June 2009

Additional Information

How to Cite

Hachulla, E., de Groote, P., Gressin, V., Sibilia, J., Diot, E., Carpentier, P., Mouthon, L., Hatron, P.-Y., Jego, P., Allanore, Y., Tiev, K. P., Agard, C., Cosnes, A., Cirstea, D., Constans, J., Farge, D., Viallard, J.-F., Harle, J.-R., Patat, F., Imbert, B., Kahan, A., Cabane, J., Clerson, P., Guillevin, L. and Humbert, M. (2009), The three-year incidence of pulmonary arterial hypertension associated with systemic sclerosis in a multicenter nationwide longitudinal study in France. Arthritis & Rheumatism, 60: 1831–1839. doi: 10.1002/art.24525

Author Information

  1. 1

    Hôpital Claude Huriez, Lille, France

  2. 2

    Centre Hospitalier Régional Universitaire, Lille, France

  3. 3

    Actelion Pharmaceuticals France, Paris, France

  4. 4

    Hôpital de Hautepierre, Strasbourg, France

  5. 5

    Hôpital Bretonneau, Tours, France

  6. 6

    Hôpital Nord, Grenoble, France

  7. 7

    Hôpital Cochin, Paris, France

  8. 8

    Hôpital Sud, Rennes, France

  9. 9

    Hôpital St. Antoine, Paris, France

  10. 10

    Hôtel Dieu, Nantes, France

  11. 11

    Hôpital Henri Mondor, Créteil, France

  12. 12

    Hôpital Central, Nancy, France

  13. 13

    Hôpital St. André, Bordeaux, France

  14. 14

    Hôpital St. Louis, Paris, France

  15. 15

    Hôpital du Haut Lévesque, Pessac, France

  16. 16

    Hôpital de la Conception, Marseilles, France

  17. 17

    Université François Rabelais, Tours, France

  18. 18

    Orgamétrie, Roubaix, France

  19. 19

    Université Paris-Sud 11, and Hôpital Antoine Béclère, Assistance Publique Hôpitaux de Paris, Clamart, France

  1. †

    Dr. Hachulla has received consulting fees, speaking fees, and/or honoraria from Actelion, Pfizer, and GlaxoSmithKline (less than $10,000 each).

  2. ‡

    Dr. Gressin owns stock or stock options in Actelion.

  3. §

    Dr. Sibilia has received speaking fees from Actelion (less than $10,000).

  4. Dr. Carpentier has received honoraria as a member of the Scientific Committee on Scleroderma for Actelion France (less than $10,000).

  5. ‖

    Dr. Mouthon has received consulting fees, speaking fees, and/or honoraria from Actelion and LFB (less than $10,000 each).

  6. ††

    Dr. Hatron has received honoraria from Pierre Fabre Laboratories and Pfizer (less than $10,000 each).

  7. ‡‡

    Dr. Allanore has received consulting fees, speaking fees, and/or honoraria from Actelion and Encysive (less than $10,000 each).

  8. §§

    Dr. Kahan has received consulting fees, speaking fees, and/or honoraria from Actelion (less than $10,000).

  9. ¶¶

    Dr. Guillevin has received speaking fees and honoraria from Actelion (less than $10,000).

*Department of Internal Medicine, National Reference Center for Scleroderma, Claude Huriez Hospital, University of Lille 2, 59037 Lille Cedex, France

Publication History

  1. Issue published online: 28 MAY 2009
  2. Article first published online: 28 MAY 2009
  3. Manuscript Accepted: 17 FEB 2009
  4. Manuscript Received: 5 AUG 2008

Funded by

  • Actelion Pharmaceuticals France

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Abstract

Objective

An algorithm for the detection of pulmonary arterial hypertension (PAH), based on the presence of dyspnea and the findings of Doppler echocardiographic evaluation of the velocity of tricuspid regurgitation (VTR) and right-sided heart catheterization (RHC), which was applied in a large multicenter systemic sclerosis (SSc) population, estimated the prevalence of PAH to be 7.85%. The aim of this observational study was to investigate the incidence of PAH and pulmonary hypertension (PH) during a 3-year followup of patients from the same cohort (the ItinérAIR-Sclérodermie Study).

Methods

Patients with SSc and without evidence of PAH underwent evaluation for dyspnea and VTR at study entry and during subsequent visits. Patients in whom PAH was suspected because of a VTR of 2.8–3.0 meters/second and unexplained dyspnea or a VTR of >3.0 meters/second underwent RHC to confirm the diagnosis.

Results

A total of 384 patients were followed up for a mean ± SD of 41.03 ± 5.66 months (median 40.92 months). At baseline, 86.7% of the patients were women, and the mean ± SD age of the patients was 53.1 ± 12.0 years. The mean ± SD duration of SSc at study entry was 8.7 ± 7.6 years. After RHC, PAH was diagnosed in 8 patients, postcapillary PH in 8 patients, and PH associated with severe pulmonary fibrosis in 2 patients. The incidence of PAH was estimated to be 0.61 cases per 100 patient-years. Two patients who exhibited a mean pulmonary artery pressure of 20–25 mm Hg at baseline subsequently developed PAH.

Conclusion

The estimated incidence of PAH among patients with SSc was 0.61 cases per 100 patient-years. The high incidence of postcapillary PH highlights the value of RHC in investigating suspected PAH.

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